scleromyxedema syndrome with involvement of eyelids

نویسندگان

مجید شمس

m shams دانشگاه علوم پزشکی شهید بهشتی- تهران- ایران محمدعلی جوادی

ma javadi دانشگاه علوم پزشکی شهید بهشتی- تهران- ایران سیدهاشم دریاباری

sh daryabari ophthalmic research center, shahid beheshti university of medical sciences, tehran, iranعلوم پزشکی بقیه اله- تهران- ایران

چکیده

purpose: to present a case of bilateral enlargement of eyelids in 29 year old man with sclromyxedema syndrome. case report: a 29- year- old man was referred to labbafinegad clinic with bilateral enlargment of upper and lower eyelids, and foreign body sensation since 9 year ago. investigating of patients history show renal deasese(hematuria ) since 9 year ago. in systemic examination the patients showed hands and feet involvement obviously. at further investhgations sclermyxderma was confirmed. conclusion: scleromyxedema is a rare, chronic and persistent idiopathic disorder characterized by a generalized papular eruption due to dermal mucin deposition with an increase in dermal collagen. uusually associated with a systemic involvement and paraproteinemia. the disease is characterized with mucin deposits in the skin and/or other organs. its etiology remains unknown affecting both genders equally between 30 and 50 years. the therapeutic options include numerous treatment modalities; however, no standard treatment exists as the rarity of this disease prevents the execution of controlled therapeutic trials.

برای دانلود باید عضویت طلایی داشته باشید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Scleromyxedema with systemic involvement mimics rheumatic diseases.

Scleromyxedema is an infiltrative skin disease produced by hyaluronic acid deposition in the dermis. A benign monoclonal gammopathy is usually present. We report 2 patients with scleromyxedema and systemic illnesses. Both patients had muscle weakness, dysphagia, and weight loss in addition to the skin changes. One also had sclerodactyly, telangiectasias, and Raynaud's phenomenon. Scleromyxedema...

متن کامل

Scleromyxedema with neurologic involvement: Therapy with intravenous immunoglobulin

Umbilical cord blood transplantation can cure pruritic eczematous eruption in a hypohidrotic ectodermal dysplasia with immunodeficiency patient Satoko Minakawa, MD, PhD, Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori, Japan; Daisuke Sawamura, MD, PhD, Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori, Japan...

متن کامل

Scleromyxedema with subclinical myositis.

A 49-year-old farmer presented with papules on the face, arms, chest and back associated with sclerosis. Histopathology and PAS stain confirmed the clinical diagnosis of scleromyxedema. He also had elevated CPK levels due to myopathy. Screening for internal malignancy was negative.

متن کامل

Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin.

Scleromyxedema is a rare disease characterized by extensive mucin deposition with fibrosis, and is associated with a monoclonal gammopathy. Currently there is no consensus on optimal treatment of this potentially fatal disease because of the lack of randomized controlled trials and limited number of case reports. At the time of this writing, 24 cases were published reporting clinical improvemen...

متن کامل

Neurological Involvement in the Course of Scleromyxedema: A Case Report.

Scleromyxedema is a rare disease of unknown etiology primarily affecting the skin, characterized by generalized papular eruption, dermal fibroblast proliferation with mucin deposition, and a monoclonal gammopathy. Neurological impairment is a rare but sometimes fatal complication of scleromyxedema that should be rapidly identified to prevent significant morbidity and mortality. A 63-year-old Ca...

متن کامل

Scleromyxedema: a cutaneous paraneoplastic syndrome associated with thymic carcinoma.

Introduction Although it is a rare disorder, scleromyxedema (lichen myxedematosus) is one of the most common forms of primary cutaneous mucinosis. This condition is a generalized form of dermal mucin deposition characterized by waxy papules that progresses to indurated and thickened skin and is often associated with monoclonal gammopathy, particularly of the immunoglobulin G type. Lichen myxede...

متن کامل

منابع من

با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید


عنوان ژورنال:
بینا

جلد ۲۱، شماره ۱، صفحات ۷۵-۷۸

میزبانی شده توسط پلتفرم ابری doprax.com

copyright © 2015-2023